SMN1 antibody (AA 80-150)

Catalog No. ABIN7270652

Product Details anti-SMN1 Antibody

Target: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Binding Specificity: AA 80-150

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This SMN1 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Purpose: SMN1 Rabbit pAb

Sequence: SQKKNTAASL QQWKVGDKCS AIWSEDGCIY PATIASIDFK RETCVVVYTG YGNREEQNLS DLLSPICEVA N

Cross-Reactivity: Human, Mouse, Rat

Characteristics: Polyclonal Antibodies

Purification: Affinity purification

Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 80-150 of human SMN1 (NP_000335.1).

Isotype: IgG

Application Details

Application Notes: WB,1:500 - 1:2000,IHC,1:50 - 1:200

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: PBS with 0.02 % sodium azide,50 % glycerol, pH 7.3.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Avoid freeze / thaw cycles.

Target Details for SMN1

Target: SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Alternative Name: SMN1 (SMN1 Products)

Synonyms: SMN2 antibody, Smn antibody, SMN antibody, BCD541 antibody, GEMIN1 antibody, SMA antibody, SMA1 antibody, SMA2 antibody, SMA3 antibody, SMA4 antibody, SMA@ antibody, SMNT antibody, T-BCD541 antibody, TDRD16A antibody, AI849087 antibody, Gemin1 antibody, SMN1 antibody, QtsA-10002 antibody, fa12d01 antibody, smn antibody, wu:fa12d01 antibody, survival motor neuron protein antibody, survival of motor neuron 1, telomeric antibody, survival motor neuron 1 antibody, survival of motor neuron 2, centromeric antibody, survival motor neuron protein-like antibody, survival motor neuron antibody, LOC461829 antibody, Smn1 antibody, SMN1 antibody, SMN2 antibody, LOC100348318 antibody, SMN antibody, LOC100713418 antibody, LOC100065744 antibody, LOC102176643 antibody, smn1 antibody

Background: This gene is part of a 500 kb inverted duplication on chromosome 5q13. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy, mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Multiple transcript variants encoding distinct isoforms have been described.,SMN1,BCD541,GEMIN1,SMA,SMA1,SMA2,SMA3,SMA4,SMN1

Molecular Weight: 27kDa/28kDa/30kDa/31kDa

Gene ID: 6606

UniProt: Q16637

Pathways: Ribonucleoprotein Complex Subunit Organization